• History and Examination
    • HPI
      • 6-month with uneventful prenatal course noted by pediatrician to have head circumference increasing from 40 to >95%ile over 2 months.
      • Child beginning to exhibit increased fussiness and delayed completion of developmental milestones
    • Exam
      • Awake, alert, interactive
      • Anterior fontanelle soft, flat
      • No splaying of cranial sutures
      • EOMI

    Initial Diagnostic Images Figure 1

    Right Vertebral Artery AP Injection Figure 2

    Right Vertebral Artery Lateral Injection Figure 3

     

    Right Vertebral Artery Lateral Injection Figure 4

    Right Vertebral Artery Lateral Injection Figure 5

     

    Post Trans-arterial Embolization of Feeding Pedicles x 3 Figure 6

    1. The well circumscribed lesion in the setting of hydrocephalus most likely represents:

    2. The Etiology of the Patient's Hydrocephalus is most likely due to which of the following:
     
    3. The next most appropriate step in management is:
    4. Supply to this malformation is primarily from:
    5. Which of the following describes you?
    6. I practice in one of the following locations.
    7. Comments
     
    • I would recommend reading an article from Dr. Lasjaunias in Neurosurgery, 2006, vol 59, no.5. Fantastic case description.
    • Please note I practice in Puerto Rico (PR).  (web App does not show that option)
    • Please send result and discussion.
    • Not iPhone friendly
    • Can this not be treated by direct embolization via the torcula

     

     

     

     

    Case Explanation:

    • HPI
      • 6-month with uneventful prenatal course noted by pediatrician to have head circumference increasing from 40 to >95%ile over 2 months.
      • Child beginning to exhibit increased fussiness and delayed completion of developmental milestones
    • Exam
      • Awake, alert, interactive
      • Anterior fontanelle soft, flat
      • No splaying of cranial sutures
      • EOMI
    • Case Description and Treatment Images
      • The term vein of Galen malformation involves a persistent fetal vessel known as the median prosencephalic vein (of Markowitz). This vein is present during the 3rd to 11th weeks of gestation and drains the majority of the diencephalic structures. As the brain develops, the anterior portion of the vein regresses with internal cerebral vein development. In healthy term infants, the internal cerebral veins drain into the posterior portion of the median prosencephalic vein, now called the “vein of Galen”.
      • In vein of Galen malformations, the anterior portion of the vein does not regress appropriately. A vein of Galen malformation therefore represents a true arteriovenous fistula.
      • Vein of Galen malformations pose several problems to the newborn. High output cardiac failure is the strongest indication for neonatal intervention. The fistula acts as a venous sump, pulling a significant portion of the infant’s circulating blood volume through the fistula. A second devastating presentation is “melting brain syndrome”.  High venous pressures can cause secondary leukomalacia and severe mental retardation. Seizures and hydrocephalus may also develop as in the presented case.  
      • Treatment may be delayed in stable infants to prevent complications from blood loss and contrast load. Delayed treatment also allows easier arterial access for endovascular treatment.
      • Treatment of complex lesions should be staged to allow a more controlled devascularization and can help prevent complications such as perfusion breakthrough and/or venous thrombosis. 

    References:

    (1) Lasjaunias P, Hui F, Zerah M, et al. Cerebral arteriovenous malformations in children. Management of 179 consecutive cases and review of the literature. Childs Nervous System 11:66-79.

    (2) Lasjaunias P, Rodesch G, Terbrugge K, et al. Vein of Galen aneurismal malformations. Report of 36 cases managed between 1982 and 1988. Acta Neurochir 99:26-37.

    (3) Mortazavi MM, Griessenauer CJ, Foreman P, et al. Vein of Galen aneurysmal malformations: critical analysis of the literature with proposal of a new classification system. J Neurosurg Pediatr. 2013 Sep;12(3):293-306.

    (4) Pearl M, Gomez J, Gregg L, Gailloud P. Endovascular management of vein of Galen aneurysmal malformations. Influence of the normal venous drainage on the choice of a treatment strategy. Childs Nerv Syst. 2010 Oct;26(10):1367-79.
     

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